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  Then came a long pause. I could sense Katsumata’s ‘Oh shit’ moment. The silence dragged on until I said, ‘Come on, Katsu. You and Richard can do it. Just get the bloody thing out.’

  Katsumata had called me from Oxford early on the Saturday morning. He returned to the bedside with Richard and they called for another echo. Reducing the pump speed allowed the left ventricle to fill and eject more blood. They asked Julie whether she felt any different and she told them she felt fine. She just wanted it out. She’d experienced no more breathlessness and there was still a normal blood pressure trace on the screen. Richard knew that the lower the speed, the higher the risk of clotting in the pump or vascular graft.

  Desiree was starting a blood transfusion and asked what I’d said on the phone.

  ‘He told me to take the pump out and don’t mention the war,’ Katsumata said with trepidation. ‘And one last thing. Only let the medical director’s office know after it’s out. We don’t want him to have a stroke.’

  ‘Then you’d better inform theatre and get on with it,’ Desiree replied.

  Richard and Katsumata explained the balance of risks to Julie and her parents. If her heart had already recovered but she bled to death from her stomach it would be a disaster. Even Richard, with his vast experience from his role in Washington, had butterflies. For him the stakes were high, as at last he was on the verge of success with the AB-180. But it was Julie’s life that really mattered.

  So Katsumata took Julie back to theatre seven days after the implant, ironically about the usual amount of time for a viral illness to get better. Richard didn’t have hospital clearance to operate so he just had to watch, although had there been an issue he’d have dived in. He had no problem with this, filled with cautious optimism at the prospect of success.

  Julie’s heart was looking good – the stiffness and swelling gone, her blood pressure stable and receiving just a little help with drugs in the background. They still had a balloon pump in reserve but she didn’t need it. Katsumata washed out the whole chest with warm saline solution, assiduously removing old blood clots from the chest cavity and from the pericardium surrounding her small but enthusiastic heart. He inserted clean chest drains, then closed the sternum tightly with wire. For the last time.

  It was important to preserve the forward momentum. Julie woke up quickly and felt much better off without the breathing machine. The tracheal tube was removed later that evening, Desiree simply ignoring her shifts and staying with her, all the while encouraging her to breathe deeply and cough despite the pain. The anticoagulation had stopped, and the blood loss from superficial stomach erosions ceased soon afterwards.

  We’d done it. We’d saved Julie’s heart.

  When Katsumata rang me with the news I’d already given my talk and was back at Johannesburg airport, homeward bound, relieved and in the mood to celebrate. Then Richard called George Magovern and his team in Pittsburgh, spreading the joy. But none of us were happier than Julie’s family, their grief and desolation lifted, and suddenly with no funeral to plan. One day soon they’d take her home, Oxford just a grim memory.

  In the 1990s any patient who received a left ventricular assist device in the United States was committed to a cardiac transplant, and few other countries had access to circulatory assist technology. What we achieved with Julie became known as ‘bridge to recovery’ in contrast to the conventional term ‘bridge to transplant’. The procedure had not been done before in the UK, and bridge to recovery – our ‘Keep Your Own Heart’ strategy – soon emerged as the preferred approach for critically ill viral myocarditis patients. I was proud of that.

  Just before Christmas 1998 the Pittsburgh engineers and researchers who’d worked on the AB-180 filed into a conference room for a special party arranged by Dr Magovern. No one knew what the occasion was – until Julie walked in with her sister. ‘The girl without a pulse’ was instantly recognisable from photographs that had been pinned to bulletin boards at the time of her groundbreaking operation, her face destined to grace the front pages. There was a moment of stunned silence, then loud cheers. Julie blushed as George shook her hand.

  ‘You being here is the best Christmas present any of us could have,’ he said.

  He was right. The company survived and thrived, and the AB-180 was modified so it could be used without opening the chest. Now called the ‘Tandem Heart’, it’s used worldwide to support patients with shock in the cardiac catheterisation laboratory.

  Julie remains well almost twenty years later, and she works in a hospital. I look forward to a reassuring card from her family every Christmas. Long may her good health last.

  8

  the black banana

  ‘We shall never surrender’

  Winston Churchill, during the Battle of Britain, 1940

  Monday 15 February 1999, 3.45 am. No one calls with good news at night. I’d been in Australia for just thirteen hours after a twenty-hour flight. In pitch darkness I scrambled across my hotel bed and knocked the receiver to the floor. The call was lost. I swiftly slipped back into sleep, courtesy of melatonin tablets and the bottle of Merlot I’d drunk at dinner. Ten minutes later the phone rang again. This time I fielded it successfully, but I was irritated.

  ‘Westaby? This is Archer. Where are you?’

  Nick Archer was the consultant paediatric cardiologist at Oxford.

  ‘Nick, you know I’m damned well in Australia. It’s the middle of the fucking night – what’s the problem?’

  I didn’t want to hear the answer.

  ‘Steve, I’m sorry, but we need you to come back. We have a sick baby with ALCAPA. The parents know you and want you to do the surgery.’

  Oh joy.

  ‘When?’

  ‘As soon as possible. She has bad heart failure and is as good as we can make her. The ventricle is poor.’

  Already there was no point in further discussion. I pictured the frantic parents, desperate for an operation before it was too late, and the four grandparents huddled around the cot trying to lend support but only transmitting anxiety. I really had no other choice.

  ‘OK, I’ll fly back today. You tell the team we’ll do it tomorrow, whenever that is.’

  It was high summer in the southern hemisphere and early-morning light had just begun to penetrate the drapes. Further attempts to sleep were pointless. I stepped through the curtains onto the balcony and gazed out at arguably the finest city vista in the world. Across the harbour the first hint of sunrise cast ghostly shadows over the Opera House. Flags fluttered on masts in the harbour below, and to my right the tall, white city lights stood out against the pink morning sky. The peace was broken by the shifting gears of a Harley-Davidson. Maybe a surgeon racing into Sydney.

  In Oxford a real-life tragedy was unfolding for this little family. Kirsty was a beautiful six-month-old baby girl in whom fate had installed a lethal self-destruct mechanism, a miserable detail that seemed destined to end her life before her first birthday. ALCAPA is an abbreviation for Anomalous Left Coronary Artery from the Pulmonary Artery, an isolated and exceptionally rare congenital anomaly in the overall complexity of human anatomy.

  Simply put, it’s bad wiring. Both coronary arteries should rise out of the aorta and supply the heart muscle with well-oxygenated blood under high pressure. They should never be attached to the pulmonary artery, as this has both low pressure and poor oxygen content. Early survival with ALCAPA therefore depends on the development of new ‘collateral’ blood vessels between the normal right coronary artery and the misplaced left coronary artery. But these eventually are insufficient to sustain blood flow to the main pumping chamber. Muscle cells deprived of oxygen die and are replaced by scar tissue, leaving baby to suffer what are in effect repeated, painful heart attacks. Scar tissue stretches, causing the left ventricle to dilate, then the heart progressively fails and the lungs become congested with blood, leading to breathlessness and exhaustion. Even during feeding.

  So by six months of age Kirsty already
had the same problem as my grandfather – heart failure through end stage coronary heart disease. But because ALCAPA is exceedingly rare, the diagnosis is seldom made until the infant is terminally ill. Fortunately, her parents were intelligent, had recognised that there was a serious problem and were persistent in finding help for her.

  Kirsty’s story was particularly harrowing. Her mother Becky already had a three-year-old son and was an experienced and responsible mum. She’d contracted no illnesses, smoked no cigarettes or drunk any alcohol during pregnancy, risking nothing that could potentially harm the foetus inside her. All antenatal checks and ultrasound scans appeared normal. Kirsty was born on 21 August 1998 by elective Caesarean section with a spinal anaesthetic and at first everything seemed normal. But not for long.

  In the womb the pressures and oxygen content of the aorta and pulmonary artery are the same, so Kirsty’s tiny heart was safe. After birth the circulations to the body and newly expanded lungs separate, and both pressure and oxygen content in the pulmonary artery fall. So, in cases of ALCAPA, both blood flow and oxygen content in that critically important left coronary artery fall precipitously, too. Kirsty was grunting even during the first attempt to breastfeed in the hospital, and Becky noticed beads of sweat trickling from the bridge of her baby’s nose. The effort of feeding repeatedly made her fractious and distressed.

  This was distinctly different from how her son had been, so Becky asked for a paediatrician to review Kirsty. She was told that there was absolutely nothing to worry about. This is exactly what anxious parents want to hear, but the truth was that no one had bothered to find out what was wrong. It was too much trouble – piss-poor medicine. At this stage Becky had little choice but to take her irritable but precious little bundle home.

  Within weeks Becky was certain that something was seriously amiss, because during every feed there was sweating and vomiting. Kirsty struggled for breath, clenched her little fists and screamed until she was puce in the face. Together they made many visits to the GP, sometimes as many as three times a week, but they always received the same non-committal reassurance – tense, unpleasant encounters, as Becky was deemed neurotic and unable to cope.

  But despite Kirsty’s rapid breathing she had no temperature. Chest infections were ruled out, and her belly was soft, with no signs of stomach or intestinal blockage. All of the common paediatric problems were excluded. Family and friends offered rational explanations – it must be colic and would get better. But with her husband working abroad Becky became more and more anxious. Kirsty wasn’t gaining weight. She had a pasty, washed-out look and a cough like a dog’s bark.

  In reality this baby was suffering repeated small heart attacks with excruciating chest pain that she could neither communicate nor understand. The human body can be outlandishly cruel.

  Eventually, after a meltdown in the GP’s surgery, Becky insisted that Kirsty should be referred to the local hospital. Twice she had chest X-rays, only to be diagnosed with bronchiolitis – inflamed breathing tubes – on both occasions. Then one day during her afternoon nap Kirsty turned a terrible slate-grey colour. She was barely rousable and limp. In abject panic Becky snatched her up and rushed to the surgery. But by the time they presented themselves at the receptionist the baby was awake and pink again. There followed yet another put-down. Becky was told to stop fussing and that there were children to be seen who were actually sick. On this occasion mother and child were acrimoniously dispatched, once more with a prescription for antibiotics. Kirsty’s enormous heart remained undetected.

  Becky’s anxiety and frustration now turned to desperation. Every instinct told her that if she didn’t push further something dreadful was going to happen, so she drove directly to the accident and emergency department of her small local hospital. They were seen by a sympathetic female doctor who had children of her own. Recognising that a mother’s instinct was usually correct, she referred them on to a larger city hospital for review by the on-call paediatrician.

  It was a bitterly cold, frosty night and they were left sitting in an unheated hospital corridor for several hours. Becky frantically struggled to keep Kirsty warm but she became progressively more limp and grey. Eventually, late into the night, they were seen. The first junior doctor suggested bronchiolitis and intended to dismiss them without investigation. Bronchiolitis seemed to be the only paediatric diagnosis these doctors had heard of. Becky was angry and frustrated, yet afraid that she would be thrown out if she protested.

  When she refused to leave without a chest X-ray she was told off for her unreasonable attitude – how thoughtless to inconvenience the hard-pressed duty radiographer at that time of night. So the sad pair were dispatched unescorted down poorly lit corridors and icy outside walkways to find their own way to the X-ray department. It was well past midnight when they returned, clutching the tell-tale picture, which Becky presented to a nurse. They were parked once more.

  Another thirty minutes passed, then a dramatic shift in attitude from the hospital staff. Kirsty and Becky were ushered into a cubicle with different doctors. Now there were hushed voices, grave expressions, and nurses bringing drips and drugs. This was even more frightening than being ignored. The previously mean, now embarrassed nurse took Becky aside to explain that Kirsty was being transferred to the specialist children’s heart unit in Oxford. By ambulance this time. Suddenly she was too sick to remain unsupervised.

  So what did the X-ray show to trigger this frenzy of activity? Kirsty had a massive heart. No one before then had bothered to examine her, but her problem was immediately obvious on the X-ray film. When the staff were pressed about her previous X-rays at the same hospital, Becky was told that the heart shadow had been misinterpreted as fluid – ‘Sorry, but it’s an easy mistake to make.’ Some mistake! How do you begin to describe a mother’s anxiety that hits like an axe, drains blood from the throat and takes your legs away?

  Things were different when they arrived in Oxford. The paediatric cardiology registrar met the ambulance and took them directly to a ward packed with children with serious heart problems and beeping monitors – a hive of activity in the depths of night.

  Nick Archer arrived at 3 am. On examining Kirsty he was immediately concerned by her body temperature. Despite Becky’s best efforts she was cold and needed to be in an incubator. An ECG and blood tests were done quickly, then the echo machine was brought in to image Kirsty’s heart chambers. First it seemed like good news. All four chambers were there, with no holes between them. But worryingly the left atrium and ventricle were both enlarged, the ventricle dramatically so. This explained the heart failure and accounted for the striking chest X-ray.

  In little more than an hour the cardiology team established that Kirsty had severe heart failure from multiple heart attacks. Parts of the left ventricular wall now consisted of thin scar tissue interspersed with poorly contracting muscle, a rare condition in an infant but one that provided the likely diagnosis. One further test was needed. A cardiac catheter would confirm the diagnosis but would require a general anaesthetic, so she’d have to be in much better condition before proceeding.

  By now, waiting in the hospital, Becky was bereft with grief, physically and emotionally drained. Her husband was away in the States on business and she felt very much alone. Guilt and irrational thoughts preoccupied her. Had she exercised too much during pregnancy? Drunk too much coffee? Offended God? There has to be a reason for everything. Deep despair took a grip, and her anxiety soon progressed to outright panic. She was certain she’d lose Kirsty. But as the winter sun crested the horizon she lost consciousness for a couple of hours. When she awoke the ward was busy, full of positive, warm-hearted people who tried to reassure her that, even though things were difficult, there was a great team caring for Kirsty.

  It was a full five weeks before Kirsty was considered fit enough for the cardiac catheter. Becky had her husband back to share the pain, and the evening before the operation Mike the anaesthetist came round to talk. Normally a jolly and
optimistic character, on this occasion Mike didn’t have much to smile about. He warned the family that Kirsty’s heart was so severely damaged that they might lose her during the procedure. It was only fair to inform them of this. So that night Kirsty was christened in her cot by the hospital chaplain, the doctors, nurses and other families gathering around the bed to support them.

  Everyone knew what the catheter would show. There was only one rare condition that did this to a baby, that caused multiple heart attacks in the first few months of life – ALCAPA. Becky overheard the words ‘early surgery’ and hoped that they didn’t mean a transplant. Both she and her husband stayed by the cot the whole night long, terrified that Kirsty might slip away. In the morning, without having slept and paralysed by fear, Becky dressed her baby in her best pyjamas and tied a bow in her hair for her trip to the catheterisation laboratory. Ironically it was Valentine’s Day. As Becky put it to me later, ‘A girl has to look her best, even when she’s poorly.’

  Once I was on the plane home I started to sketch the anatomy of Kirsty’s aorta, pulmonary artery and abnormal left coronary artery. I knew that the current techniques in operations for ALCAPA had limitations and a substantial failure rate, so I used my time during the flight to work out an alternative. By the time we were cruising high above Java I’d designed my new operation. Last on, I was first off the plane back in London. As I waited for the airbridge to connect and the doors to open, the cabin services director handed me a bottle of champagne, wished me luck and whispered, ‘You operated on my sister’s baby.’ Small world. I thanked her.

  I called my colleague Katsumata when I got back to Oxford and asked him to bring Kirsty’s parents to my office with a consent form. The cardiac catheter had shown precisely what Archer had suspected. Kirsty needed surgery as soon as possible.

  Becky looked tired and drawn when I saw her. She knew instinctively who I was. Her face lit up as they entered my office in a cold Portakabin.